ABSTRACT
INTRODUCTION: Pseudomyxoma peritonei (PMP) is characteristically divided into two histopathological subtypes; disseminated peritoneal adenomucinosis (DPAM) and peritoneal mucinous carcinomatosis (PMCA). The latter is associated with a worse prognosis. However, even within the DPAM group, there is a considerable variation in outcome. In this study we investigate the role of baseline serum tumor markers CA 19-9, CEA and CA-125 in further stratifying survival. METHODS: Over 16 years, 218 patients with PMP were treated with cytoreductive surgery (CRS) and perioperative intraperitoneal chemotherapy (PIC) at our institution. A CA-125 level of >35 U/L, CA 19-9 of >40 U/mL and CEA of >3 ng/mL were considered positive or elevated outside the laboratory reference range. The impact of clinicopathologic and treatment-related variables on overall survival (OS) was analyzed with the Kaplan Meier method. Survival curves were compared using the log-rank test. Variables deemed significant by univariate analyses were entered into multivariate analysis using the Cox proportional hazards model. RESULTS: Within the DPAM group, the 5-year survival of patients who were CA 19-9 positive versus those with normal values were 58% and 90% respectively (P<0.001). Other variables found to negatively impact on OS in univariate analyses were completeness of cytoreduction (CC) score 2/3 (P<0.001), peritoneal cancer index (PCI) >25 (P<0.001) and male gender (P=0.017). In the Cox regression model, only CA 19-9 positivity was found to be an independent prognostic factor for OS (P=0.034). In addition to marker positivity, the absolute level of CA 19-9 was also prognostically significant. In patients with CA 19-9>1,000 U/mL, the 5-year survival was 23%, in contrast to 90% in patients with CA 19-9<100 U/mL (P<0.001). In the PMCA cohort, only CC-score was found to be associated with OS (P<0.001). CONCLUSIONS: Our study provides relevant prognostic information for the DPAM subtype in staging and prioritizing surgery; as even in apparently indolent disease, some patients have poorer survival. CA 19-9 elevation may also be useful in identifying patients who would potentially benefit from adjuvant therapy and/or closer post-operative surveillance. The potential role of CA 19-9 in mediating tumor cell adhesion and disease progression in PMP should be further investigated to deepen our understanding of the disease's inherent biological behavior. If a true relationship exists, CA 19-9 may be a conceivable target for immunotherapy.
ABSTRACT
BACKGROUND: Small bowel adenocarcinoma is a rare malignancy that presents both a diagnostic and therapeutic challenge. The late presentation is often associated with disseminated carcinomatosis which is regarded a terminal event. We review our experience with small bowel peritoneal carcinomatosis following treatment with cytoreductive surgery (CRS) and perioperative intraperitoneal chemotherapy (PIC). METHODS: From a prospective database of CRS and PIC, seven patients were identified to have undergone treatment for small bowel peritoneal carcinomatosis with CRS and hyperthermic intraperitoneal chemotherapy (Mitomycin C) and early postoperative intraperitoneal chemotherapy (5FU). A retrospective review was undertaken to describe the clinicopathological characteristics and survival outcomes. RESULTS: The median follow-up was 17 months (range, 5-46 months). Six of seven patients have died. The disease-free survival was 12 months and the overall median survival was 25 months. The 1-, 2-, and 3-year survivals were 57%, 38%, and 20% respectively. Tumor histology of poorly differentiated adenocarcinoma with signet ring, lymphovascular invasion and perineural invasion appeared to be associated with a poor outcome. CONCLUSION: Cytoreductive surgery and perioperative intraperitoneal chemotherapy is a treatment option for small bowel cancer peritoneal carcinomatosis with encouraging survival results.
Subject(s)
Adenocarcinoma/therapy , Hyperthermia, Induced , Ileal Neoplasms/therapy , Jejunal Neoplasms/therapy , Adenocarcinoma/mortality , Adult , Combined Modality Therapy , Female , Humans , Ileal Neoplasms/mortality , Jejunal Neoplasms/mortality , Male , Middle Aged , Peritoneal Neoplasms/mortality , Peritoneal Neoplasms/therapy , Prospective StudiesABSTRACT
Peritoneal Cancer Index (PCI) has been recognized as an independent prognostic indicator for long-term outcomes. It also influences the likelihood of complete cytoreduction, another principal determinant of long-term survival. The objective of this study was to evaluate the utility of preoperative CT in estimating PCI during the patient selection process. The efficacy of CT in demonstrating peritoneal disease was evaluated by comparing the radiological and intraoperative lesion size and PCI scores using the Wilcoxon signed-rank test. Tumor distribution was assessed in each abdominopelvic region as tumor present versus absent. The sensitivity, specificity, positive predictive value, and negative predictive value were calculated in each abdominopelvic region. Overall, where CT identifies the presence of disease, it portrayed lesion size accurately in 60%, underestimated in 33%, and overestimated in 7% of cases. Analysis of individual abdominopelvic regions demonstrated a statistically significant difference between radiologically and intraoperatively visualized lesion sizes (P < 0.05) except in the epigastrium, left upper, and left flank regions. The sensitivity of CT in detecting peritoneal implants was influenced by lesion size. Small nodules (<0.5 cm) were visualized on CT with only a sensitivity of 11%, which is in contrast to 94% with nodules exceeding 5 cm. Radiological PCI scores significantly underestimated intraoperative PCI (P < 0.001). This study demonstrated that the sensitivity of CT in detecting peritoneal implants was influenced by lesion size and CT PCI significantly underestimated clinical PCI. The role of CT in refining patient selection and improving prognosis remains to be closely evaluated.
